Epidural angioleiomyoma: an extraordinary cause of compressive myelopathy-MRI findings with histopathological correlation.

BACKGROUND: Angioleiomyomas are benign mesenchymal tumors usually located in the limbs, with anecdotal reports in the spine. We present an atypical case of an epidural spine angioleiomyoma presenting with compressive myelopathy symptoms. The diagnosis was suggested based on MRI findings, and subsequently confirmed histopathologically. RESULTS: This is the first known occurrence of pure spinal epidural angioleiomyoma as a source of compressive myelopathy. The imaging presentation, especially the 'dark reticular sign' on MRI, was crucial in suggesting the diagnosis despite the atypical location CONCLUSION: This report serves to raise awareness among clinicians and radiologists about including angioleiomyoma in differential diagnoses for spinal epidural lesions with indicative MRI features. The favorable outcome after surgical intervention underscores the necessity of swift and accurate diagnosis followed by appropriate treatment for such uncommon spinal tumors.

Massive spinal epidural infantile hemangioma, image findings, and treatment: a case report and review of literature.

Spinal involvement of infantile hemangiomas is rare with the predilection to involve the epidural space. A proper diagnosis might be challenging due to the atypical location and variable/inconsistent use of the International Society for the Study of Vascular Anomalies (ISSVA) classification by radiologists, pathologists, and clinicians. A proper diagnosis of epidural infantile hemangioma is key due to the different aggressiveness of the treatment options with inconstant literature regarding the best available treatment. Herein, we present a case of a massive epidural infantile hemangioma successfully treated with only beta-blocker. We discuss the clinical, MRI, CT, ultrasound, and histological features of this lesion as we review the literature with the objective of addressing some of the confusion surrounding the subject.

What Predicts the Prognosis of Spinal Metastases in Separation Surgery Procedures?

BACKGROUND: Separation surgery is performed to provide a safe gap between the epidural tumor and spinal cord for postoperative stereotactic body radiotherapy (SBRT) in cases of spinal metastases. However, there is a gap in evidence regarding sufficient tumor resection in separation surgery. We describe the prognoses according to the extent of resection in separation surgery. METHODS: This retrospective study included 36 consecutive patients who underwent separation surgery and postoperative SBRT between December 2016 and December 2019 at a single center. Local control (LC), overall survival (OS), distance of separation (DS), and quality-of-life parameters were analyzed. P values <0.05 were considered statistically significant. RESULTS: Patients were assigned to the aggressive resection group (ARG, n = 18) or moderate resection group (MRG, n = 18), with estimated LC and OS at 1 year of 79.0% and 75.9%, respectively. There were no significant differences between ARG and MRG in estimated LC (85.9% vs. 72.2%; P = 0.317) or OS (69.3% vs. 80.9%, P = 0.953) at 1 year. All 5 patients in MRG who developed local progression had less satisfactory tumor resection with DS <3 mm. A borderline significant difference in estimated LC at 1 year was noted between individuals with DS <3 mm and those with DS ≥3 mm (51.9% vs. 100.0%; P = 0.053) in MRG. There was no statistical difference between ARG and MRG in quality-of-life parameters. CONCLUSIONS: Moderate resection of ventral dural mass did not significantly reduce patients' prognosis in separation surgery. However, the minimal distance between the postoperative residual epidural tumor and spinal cord should be ≥3 mm.

A pilot study of stereotactic boost for malignant epidural spinal cord compression: clinical significance and initial dosimetric evaluation.

PURPOSE: Metastatic epidural spinal cord compression (MESCC) is a devastating complication of advanced malignancy, which can result in neurologic complications and significant deterioration in overall function and quality of life. Most patients are not candidates for optimal surgical decompression and as a result, receive urgent 3D conformal radiotherapy (3DCRT) to prevent or attempt to reverse neurologic progression. Multiple trials indicate that response and ambulatory rates after 3DCRT are inferior to surgery. The advent of stereotactic body radiation therapy (SBRT) has created a method with which a "radiosurgical decompression" boost may facilitate improve outcomes for MESCC patients. METHODS: We are conducting a pilot study to investigate SBRT boost after urgent 3D CRT for patients with MESCC. The aim of the study is to establish feasibility of this two-phase treatment regimen, and secondarily to characterize post-treatment ambulation status, motor response, pain control, quality of life and survival. DISCUSSION: We describe the study protocol and present a case report of one patient. A quality assurance review was conducted after the first seven patients, and resultant dose-constraints were revised to improve safety and feasibility of planning through more conservative organ at risk constraints. There have been no severe adverse events (grade 3-5) to date. We have illustrated clinical and dosimetric data of an example case, where a patient regained full strength and ambulatory capacity. CONCLUSIONS: Our study aims to determine if SBRT is a feasible option in addition to standard 3DCRT for MESCC patients, with the goal to consider future randomized trials if successful. Having a robust quality assurance process in this study ensures translatability going forward if future trials with multicenter and increased patient representation are to be considered. TRIAL REGISTRATION: clinicaltrials.gov; registration no. NCT03529708; https://clinicaltrials.gov/ct2/show/NCT03529708 ; First posted May 18, 2018.

A movement disorder specialist gets a taste of his own medicine.

We present a case in which a movement disorder neurologist developed two different hyperkinetic movements due to corticospinal tract hyperexcitability, which resolved completely with surgical removal of an epidural spinal canal tumor. The first-hand description of these movements creates a unique opportunity for enhanced insight into these complex movement phenomena.

Outcome of Epidural Hematoma: Lessons from Solitary Fibrous Tumor/Hemangiopericytoma.

BACKGROUND: Intracranial solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is rare. In this report, a case of epidural hematoma (EDH) that eventually evolved into SFT/HPC is presented. We describe the possible association between the 2 diseases, which has not been previously reported. CASE DESCRIPTION: A 40-year-old man suffered from an EDH in the right parietal area 12 years ago and accepted conservative treatment. Follow-up computed tomography (CT) scan shows that the density of the right EDH gradually changed from uniform slightly lower density to mixed density. A new CT scan revealed an epidural mass extending to the subcutaneous with local bone destruction. An operation was performed via a large right parietal craniotomy, and the final diagnosis was World Health Organization grade III SFT/HPC after histopathologic examination and immunohistochemical verification. The patient died of deterioration of brain disease 3 months after the final diagnosis. CONCLUSIONS: To our knowledge, this is the first report that HPC occurred in the epidural cavity. We are the first time to describe the possible association between EDH and HPC.

The clinical utility of the Spinal Instability Neoplastic Score (SINS) system in spinal epidural metastases: a retrospective study.

STUDY DESIGN: A retrospective study. OBJECTIVES: This study assessed the clinical utility of the Spinal Instability Neoplastic Score (SINS) in relation to the surgical treatment of spinal epidural metastasis and factors important for surgical decision-making. These factors include epidural spinal cord compression (ESCC), patient prognosis and neurologic status. SETTING: Queen Elizabeth II Health Sciences Centre, Halifax, Canada. METHODS: We identified 285 patients with spinal metastatic disease. Data were extracted through a retrospective review. SINS and ESCC were scored based on CT and MRI, respectively. RESULTS: Patients were grouped into stable (35%), potentially unstable (52%), and unstable (13%) groups. The overall incidence of metastatic spinal deformity was 9%. Surgical interventions were performed in 21% of patients, including decompression and instrumented fusion (70%), decompression alone (17%), percutaneous vertebral augmentation (9%), and instrumented vertebral augmentation (5%). The use of spinal instrumentation was significantly associated with unstable SINS (p = 0.005). Grade 3 ESCC was also significantly associated with unstable SINS (p < 0.001). Kaplan-Meier analysis revealed that SINS was not a predictor of survival (p = 0.98). In the radiotherapy-alone group, a significant proportion of patients with potentially unstable SINS (30%) progressed into unstable SINS category at an average 364 ± 244 days (p < 0.001). CONCLUSION: This study demonstrated that more severe categories of SINS were associated with higher degrees of ESCC, and surgical interventions were more often utilized in this group with more frequent placement of spinal instrumentation. Although SINS did not predict patient prognosis, it correlates with the progression of metastatic instability in patients treated with radiotherapy.

Cervical-Thoracic Epidural Giant Cell Reparative Granuloma: Case Report and Review of the Literature.

BACKGROUND: Giant cell reparative granuloma (GCRG) is a rare benign tumor. The jawbone is the most common site of occurrence, followed by sphenoid bone, craniofacial bone, hand and foot bones. The etiology of GCRG is unknown but may be related to an intraosseous hemorrhage following trauma. Despite its benign nature, it could be locally aggressive. To our knowledge, no spinal epidural GCRG case has been reported. CASE DESCRIPTION: A case of man aged 32 years who presented with upper right limb numbness and weakness. Computed tomography showed a round soft tissue mass in the spinal canal at the C7-T1 level. The mass showed isointensity on T1-weighted images, hypointensity on T2-weighted images, and significant enhancement on postcontrast T1-weighted images. The mass localized in the epidural space and was surgically resected. The histologic diagnosis was consistent with GCRG. CONCLUSIONS: Spinal epidural GCRG is rare and is hardly considered in the differential diagnosis. Preoperative diagnosis of GCRG is challenging, and the definitive diagnosis could only be made by pathological examination. Surgical resection is probably an effective therapy for relief of symptoms.

Hematolymphoid Malignancies Presenting with Spinal Epidural Mass and Spinal Cord Compression: A Case Series with Rare Entities.

The epidural space is an uncommon site for involvement by hematolymphoid malignancies, and may present unexpectedly with neurological symptoms related to spinal cord compression. Our objective was to review the clinical and pathologic features of cases with initial presentations of cord compression, subsequently diagnosed as a hematolymphoid malignancy after pathologic examination. Review of the Department of Pathology's archives revealed 15 patients who presented with spinal cord compression due to epidural hematolymphoid malignancies between 2008-2019. These cases involved five primary epidural lymphomas, including an ALK-negative anaplastic large T-cell lymphoma previously not reported at this site, three diffuse large B cell lymphomas, one B-lymphoblastic lymphoma, four cases of myeloid sarcoma, one case with a previous history of acute myeloid leukemia, five cases with plasma cell neoplasms and epidural lesions as the initial presentation of plasma cell myeloma, one case showing aberrant T-cell marker expression, and one case being a histiocytic sarcoma that is rarely reported in the spine. A hematolymphoid malignancy was suspected clinically or radiologically in only five of these cases. These cases represent the spectrum of hematolymphoid malignancies that can involve the epidural space and present for the first time with cord compression, resulting in clinical, radiological and pathologic diagnostic challenges. Their diagnoses require a high degree of awareness, suspicion, and thorough histologic evaluation with ancillary studies for appropriate disease classification and therapeutic intervention. To our knowledge, this is one of the largest and most diverse of such series in the English language literature.

Pure Lumbar Epidural Cavernous Hemangioma with Contralateral Symptoms Complicated by Spondylolisthesis.

Pure spinal epidural cavernous hemangioma with contralateral symptoms is rarely reported. It will be very difficult to diagnose when it is complicated by contralateral symptoms. A 56-year female presented with pure lumbar epidural cavernous hemangioma with contralateral symptoms. Physical examination and imaging including X-ray, CT and MRI, suggested that it is a hemangioma complicated by spondylolisthesis. Laminectomy and exploration was performed. Postoperative X-ray showed satisfactory interbody fusion and reduction of spondylolisthesis. The patient was fully recovered four months postoperatively. Histopathological examination showed that the mass was epidural cavernous hemangioma.

Clinical efficacy of computed tomography-guided iodine-125 seed implantation therapy for patients with metastatic epidural spinal cord compression: A retrospective study.

BACKGROUND: This study evaluated the clinical efficacy of computed tomography (CT)-guided radioactive iodine-125 (125 I) seed implantation in patients with metastatic epidural spinal cord compression (MESCC). MATERIALS AND METHODS: A cohort of 22 patients with MESCC were retrospectively enrolled. All patients underwent CT-guided 125 I seed implantation therapy via standard procedures. Clinical indexes, including the University of Texas MD Anderson Cancer Center (MDA) criteria for tumor responses, numerical rating scale (NRS) for the degree of pain, Karnofsky Performance Status (KPS) for quality of life, American Spinal Injury Association (ASIA) impairment scale, grade of ESCC, and radiation dose, were evaluated and recorded pre- and post-operation. A follow-up evaluation was performed at least 3 months after the operation. Finally, pre- and post-operative differences in these clinical indexes were compared. Overall survival was recorded. RESULTS: Operations were successfully performed on all patients. A median of 48 (range, 7-103) seeds were implanted in lesions, and the postoperative target verified dose D90 was 11,072.4 ± 1773.5 cGy. Patients were followed for a median of 6 months (range, 3-38 months). The median survival time was 10 months; the response rate was 18/22 (82%); the local control rates at 3, 6, and 12 months were 91.3%, 81.9%, and 81.9%, respectively; and the survival rates were 80%, 50.0%, and 21.9% at 6, 12, and 18 months, respectively. The ESCC grade was significantly lower (P < 0.05). Based on the ASIA impairment scale, the nerve functional reservation, recovery, and decline rates were 63.7% (14/22), 27.3% (6/22), and 9% (2/22), respectively. The NRS and KPS were both significantly improved in the 3rd month of follow-up (P < 0.05). CONCLUSION: CT-guided 125 I seed implantation represents an effective and safe palliative care for patients with MESCC, which can effectively relieve pain and spinal cord compression and improve nerve function and quality of life.

Spinal angiolipomas in pregnancy: Natural history and surgical treatment.

Spinal angiolipomas (SALs) are rare extradural tumors frequently located at the level of the thoracic spine and they are associated with spinal cord compromise that might result in severe myelopathy. While the first macroscopic description dates 1890, histologically these tumors where not described as angiolipomas until 1986 by Haddad et al. Occurrence in pregnancy is even more infrequent. Since their first macroscopic description, spinal angiolipomas were reported anecdotally in pregnant women. We present a case of spinal angiolipoma in pregnancy with confirmed histologic diagnosis. In the present paper, we reviewed the literature regarding spinal angiolipomas in order to characterize their clinical manifestation, natural history, radiologic and histologic appearance. We add also a further case of spinal angiolipoma in a pregnant woman. Finally, we provide suggestions for the management of such rare tumors in pregnancy.

Spinal Epidural Venous Angioma Presenting Symtomps of Lumbar Disc Herniation: A Case Report.

Spinal epidural venous angiomas are extremely rare. We report the case of a 60-year-old man who presented with disc herniation symptoms, complaining of pain in his left leg and numbness, especially at the base of the knee. On physical examination, the Lasègue test was positive at 45 degrees on the left side and no neurological deficit was detected on the lower extremity. Contrast enhanced magnetic resonance imaging revealed a lesion in the left S1 neural foramen, which was initially evaluated as a schwannoma. However, after the lesion was totally excised, it was pathologically identified as a venous angioma. Here, the clinical presentation, management, and surgical, radiological, and pathological features are discussed.

Tumor-to-tumor metastasis of lung adenocarcinoma into a spinal intradural solitary fibrous tumor: a case report.

Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms commonly involving visceral or parietal pleura. We present the first report of tumor-to-tumor metastasis involving a pulmonary adenocarcinoma donor and an intradural SFT recipient. The patient presented with a 1 year history of diffuse back pain. A spinal intradural contrast-enhancing mass at the T9/10 level and a tumor of the lung were diagnosed radiologically. Bronchoscopic biopsy confirmed pulmonary adenocarcinoma in the right upper lung lobe. Due to deteriorating neurological status with conus medullaris syndrome, we performed a neurosurgical excision of the lesion. Histological analysis of the tumor revealed tumor-to-tumor metastasis of the adenocarcinoma to the SFT.

Rare Case of Dumbbell-Shaped Spinal Cavernous Hemangioma and Literature Review.

BACKGROUND: Spinal epidural cavernous hemangiomas are rare vascular malformations. Exceptionally, they present with dumbbell-shaped morphology. When they happen, it's mandatory to include their pathology in the differential diagnosis because of their similarity to schwannomas. CASE DESCRIPTION: We report the case of a 72-year-old woman with a dumbbell-shaped thoracic epidural cavernous hemangioma. A literature review of diagnostic features and current treatment options are also discussed. CONCLUSIONS: Surgery is safe and effective in both improving patient condition and preventing acute hemorrhage that can worsen the outcome, causing neurologic and potentially irreversible deficits. The favorable result we obtained in our patient suggests that surgery should be evaluated as the first option, even in patients with large epidural cavernous hemangiomas.

Intracranial Epidural Metastases of Adrenal Pheochromocytoma: A Rare Entity.

BACKGROUND: Pheochromocytomas are uncommon neuroendocrine tumors of the adrenal medulla. Malignant behavior is seen in approximately 10% of these lesions, evidenced by distant metastasis to sites without chromaffin tissue. Here we report a rare case of intracranial epidural metastases of an adrenal pheochromocytoma in a 24-year-old man. CASE DESCRIPTION: The patient originally presented at age 10 years with adrenal pheochromocytoma and subsequently developed extensive metastatic bone and lung disease. He was monitored in the intervening years until recent imaging demonstrated an enlarging right parietal mass. On surgical resection of the parietal lesion, the tumor was highly vascularized and confined to the epidural space. CONCLUSIONS: To the best of our knowledge, this is the first reported case of metastatic epidural spread of pheochromocytoma without concomitant subdural or intraparenchymal extension.

Sudden paraplegia due to spontaneous bleeding in a thoracic epidural angiolipoma and literature review.

BACKGROUND: Spinal angiolipomas are rare epidural tumours that are usually revealed by chronic symptoms of medullar irritation. We report a case of acute paraplegia caused by spontaneous bleeding revealing a thoracic angiolipoma. CASE DESCRIPTION: A 17-year-old male patient with no previous medical history was admitted for acute onset of paraplegia with bladder retention and loss of sensation in the lower limbs, preceded by dorsal pain during the three previous days. MRI showed an enhanced T1-weighted image of a T7-T12 epidural lesion. The T1-weighted isosignal and the T2-weighted hyposignal suggested haemorrhagic complications. Due to a mass effect on the spinal cord, an emergency laminectomy was performed. Histopathological examination of the lesion revealed an angiolipoma with spontaneous bleeding. Clinical outcome was favourable after two months. CONCLUSION: This case is one of the first to be reported, although the clinical presentation is similar to that of other rare reported cases of paraplegia due to spinal compression by tumoural bleeding.

Purely Spinal Epidural Capillary Hemangiomas.

Capillary hemangiomas (CAs) are benign endothelial cell neoplasms that are often encountered superficially in the soft tissues of the head and neck region. Most of the reported purely spinal epidural hemangiomas have been of cavernous type, and the occurrence of purely spinal epidural CA is exceedingly rare, and there are only 12 reported cases of spinal epidural CAs in the English literature. Herein, the authors report the 13th case of purely spinal epidural CAs, and the clinical characteristics, histopathological features, and treatment were also investigated.

Imaging Appearances and Pathologic Characteristics of Spinal Epidural Meningioma.

BACKGROUND AND PURPOSE: Spinal epidural meningioma is an uncommon tumor. This study aimed to analyze the imaging and pathologic characteristics of this rare tumor. MATERIALS AND METHODS: Fourteen confirmed cases of epidural meningioma were retrospectively reviewed, and imaging characteristics and pathologic findings were analyzed to identify the typical features. RESULTS: The mean age of the patients (4 men, 10 women) was 44.9 years. Twelve tumors were in the cervical spinal canal, and 2, in the thoracic spinal canal. There were 9 en plaque meningiomas, 4 dumbbell-shaped meningiomas, and 1 fusiform/ovoid meningioma. The epidural meningiomas extended over 2-5 spinal segments (mean, 3.2 spinal segments). A soft epidural mass was seen in 12/14 (86%) patients. Dural calcification was seen in 8/14 (57%) tumors. Tumor caused intervertebral foramen enlargement in 10/14 (71%) patients and adhered to the nerve roots in 11/14 (79%) patients. Intradural invasion was seen in 8/14 (57%) patients. The dural tail sign was present in 13/14 (93%) tumors on contrast-enhanced T1WI. Regarding pathologic type, 10 of 14 (71%) were psammomatous, 2 of 14 (14%) were meningothelial, 1 of 14 (7%) was angiomatous, and 1 of 14 (7%) was transitional. During follow-up (mean follow-up, 73.4 months; range, 4-192 months), 7 patients had recurrence. Recurrences were between 4 and 192 months after the operation. CONCLUSIONS: Epidural meningioma has 3 different growth patterns. Dural thickening, calcification, invasion, and epidural mass formation are characteristic features of epidural meningioma. Regular follow-up imaging is required to detect recurrence.